Is there a role for the otolaryngologist in PFAPA syndrome? A systematic review

Abstract

Aims: To define the rote of medical or surgical treatment in patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA syndrome). Methods: A Medline search was performed using the terms PFAPA, periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis, treatment. A systematic review of the English literature was performed. Papers on pyrexia of unknown origin in the pediatric population were excluded; this was because the clinical entities included in the differential diagnosis of pyrexia of unknown origin share many clinical characteristics with PFAPA, and may be ill defined. Results: Twenty-seven papers have been published since 1989, of which 20 were in English language. There were five single case reports and two papers involving two patients each. There were 6 retrospective reviews of case notes, involving 5-94 patients over a 3-10 year review period. Given that current evidence on the effectiveness of tonsillectomy in PFAPA is extremely weak (level of evidence V), tonsillectomy should not be performed. Conclusions: PFAPA usually resolves without any long-term adverse effect, and as such, there is no rote for tonsillectomy in these patients. (C) 2006 Elsevier Ireland Ltd. All rights reserved.